Learning objectives

• Describe Sjogren’s syndrome
• Recognize the symptoms and signs of Sjogren’s syndrome
• Anesthetic management of a patient with Sjogren’s syndrome

Definition and mechanisms

• Sjogren’s syndrome is a frequent autoimmune disease (autosomal recessive disorder) characterized by dysfunction and destruction of exocrine glands (e.g., lacrimal and salivary glands), associated with lymphocytes infiltrates and immunological hyperreactivity
• The hallmark symptoms of Sjogren’s syndrome are dry eyes and a dry mouth
• The condition often accompanies other immune system disorders (e.g., rheumatoid arthritis, systemic lupus erythematosus, and multiple sclerosis)

Signs and symptoms

• Dry eyes (keratoconjunctivitis sicca)
• Dry mouth (xerostomia)
• Joint pain, swelling, and stiffness
• Swollen salivary glands
• Skin rashes or dry skin (xeroderma)
• Vaginal dryness
• Chronic dry cough
• Prolonged fatigue
• Thyroid problems

Causes

• Genetic factors: Mutations in HLA-DR and HLA-DQ genes
• Environmental factors: Viruses (e.g., Epstein-Barr virus, hepatitis C, and human T-cell leukemia virus type 1), hormones (e.g., estrogen, prolactin), etc.

Complications

• Dental cavities
• Yeast infections
• Vision problems: Light sensitivity, blurred vision, and corneal damage
• Lymphoma
• Inflammation can cause pneumonia, bronchitis, or other problems in the lungs; lead to problems with kidney function; and cause hepatitis or cirrhosis of the liver
• Peripheral neuropathy (numbness, tingling, and burning of the hands and feet)

Risk factors

• Age >40 years
• Female gender
• Rheumatic disease (i.e., rheumatoid arthritis or lupus)

Pathophysiology

Treatment

• Medications
  • Decrease eye inflammation (artificial tears): Cyclosporine or lifitegrast
  • Increase production of saliva (and sometimes tears): Pilocarpine and cevimeline
  • Address specific complications: NSAIDs or other arthritis medications, antifungal medications for yeast infections
• Surgery to seal the nasolacrimal ducts that drain tears from the eyes 

Management

Preoperative management

• Complete medical history: Evolution and symptomatology of the disease
• Thorough physical examination
• Continue chronic therapy until the morning of surgery

Intraoperative management

• Goal: Humidify the mucous membranes rigorously during the anesthetic procedure
• Lubricate the eyes to prevent corneal damage
• Avoid respiratory dryness by using humidified oxygen
• Suction efficiently to prevent mucous plugs
• Avoid parasympatholytic and anticholinergic drugs due to their decrease in gland secretions

Postoperative management

• No increased risk for postoperative complications

Suggested reading

• Sjögren Syndrome. In: Bissonnette B, Luginbuehl I, Marciniak B, Dalens BJ. eds. Syndromes: Rapid Recognition and Perioperative Implications. McGraw Hill; 2006. Accessed February 20, 2023. https://accessanesthesiology.mhmedical.com/content.aspx?bookid=852&sectionid=49518220

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